title: Potential role of thrombelastography in the monitoring of acquired factor VIII inhibitor hemophilia A: report on a 78-year-old woman with life-threatening bleedings.
abstract: A 78-year-old woman was admitted to our hospital because of syncope associated with hematomas in both legs. Acquired hemophilia A (AHA) with a low antifactor VIII antibodies activity was diagnosed. Whole blood (WB) thrombelastographic profile depicted a hypocoagulable state. During hospitalization, the patient experienced life-threatening bleedings in the neck and in the right thigh. FVIII concentrates and rFVIIa was safe and effective in controlling acute hemorrhagic symptoms. Immunosuppressive therapy was used successfully to eradicate the inhibitor. At discharge, FVIII inhibitor was absent and thrombelastogram showed a normal profile. Our report confirms that AHA is a heterogeneous condition in terms of risk of bleeding. Even though the criteria for the diagnosis of AHA is quite well defined, a laboratory test useful to predict the bleeding risk and monitor the response to treatment is lacking. ROTEM profile appears to be correlated with the response to treatment and with the eradication of the inhibitor.
authors.affiliation: Department of Medical and Surgical Sciences, 2nd Chair of Internal Medicine, University of Padua Medical School, Via Ospedale 105, Padua, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Spiezia
authors.forename: Leonardo
authors.initials: L
authors.lastname: Meneghetti
authors.forename: Fabio
authors.initials: F
authors.lastname: Dalla Valle
authors.forename: Giulio
authors.initials: G
authors.lastname: Tognin
authors.forename: Claudia
authors.initials: C
authors.lastname: Radu
authors.forename: Graziella
authors.initials: G
authors.lastname: Saggiorato
authors.forename: Mariangela
authors.initials: M
authors.lastname: Fadin
authors.forename: Ezio
authors.initials: E
authors.lastname: Zanon
authors.forename: Paolo
authors.initials: P
authors.lastname: Simioni
mesh_terms: D000208:Acute Disease; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D016903:Drug Monitoring / Q000379:methods; D005169:Factor VIII / Q000037:antagonists & inhibitors / Q000276:immunology*; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000276:immunology*; D006470:Hemorrhage / Q000097:blood / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011994:Recombinant Proteins / Q000627:therapeutic use; D012720:Severity of Illness Index; D013916:Thrombelastography
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa